[BIO] VITAMIN DEFICIENCY Flashcards
thiamine function
forms TPP by an ATP dependent enzyme in liver
reactions requiring TPP
1- oxidative decarboxylation of α-ketoacids
2- transketolase reactions
3- decarboxylation of pyruvic acid in yeast
4-branched chain AA catabolism
oxidative decarboxylation of α-ketoacids
pyruvate to Acetyl CoA
α-ketoglutarate to succinyl CoA
branches
decarboxylation of pyruvic acid
pyruvate to Acetylaldehyde
Thiamin enzymes (4)
1- PDH
2-α-ketoglutarate dehydrogenase
3- Transketolase
4- Branched-chain ketoacid dehydrogenase
which pathways is Thiamine involved in?
PDH
TCA
HMP
Branched-chain AA catabolism
Thiamin deficiency causes
decreases pyruvate & ketoglutarate dehydrogenase activity—>decreased ATP–> impaired cellular functions
diseases associated w/ thiamin deficiency
Beriberi= polished rice diet
Wernicke-Korsakoff syndrome= seen in alcoholics
why is Wernicke-Korsakoff syndrome commonly found in alcoholics?
alcohol impairs Vit B1 intestinal absorption
biochemical tests of thiamine status (5)
1- Blood thiamine
2- Erythrocyte transketolase activity
3- Thiamine pyrophosphate effect
4- Urinary thiamine
5-Blood pyruvate & lactate levels
PDC role
converts pyruvate to acetyl CoA
to produce citrate
PDH deficiency
1- pyruvate-> acetyl CoA pathway interrupted
2- pyruvate shunted to produce lactic acid + alanine
3- citrate production= limited
4- citric acid= can’t proceed
how is acetyl-CoA produced in case of PDH deficiency
alternate metabolic pathways are stimulated but energy deficit remains in CNS
clinical picture of PDH deficiency (7)
lactic acidosis
hypotonia
lethargy
poor feeding
structural abnormalities in brain
seizures
neuropathological spasms
symptoms of PDH usually progress to
mental retardation
microcephaly
blindness
spasticity